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Objectives Our study aimed to reveal the frequency of patient safety incidents (PSI) in dentomaxillofacial radiology (DMFR), including their mitigating and contributing factors, to help recognize and thus better prevent these adverse events (AE) in the future. Methods Hospital District Helsinki and Uusimaa (HUS) and the City of Helsinki (HKI) use HaiPro, an anonymous web-based tool, for healthcare professionals to report PSI. Dentistry-related PSIs were evaluated individually to find any DMFR-related reports. Additionally, we searched the HaiPro-data using multiple dentistry- and DMFR-related keywords. We compartmentalized all DMFR-related PSI by their type and assessed their contributing factors, as well as their risk classification, severity, outcome, and possible corrective actions. Results In HUS and HKI, 43 of the 195,589 HaiPro-reports filed during 2012–2017 were DMFR-related. The most prevalent event type of DMFR-related PSIs was laboratory-, medical imaging-, or other patient examination-related events (33%). The second most common event type was defined as being related to flow or control of information (26%). For both of these event types, the most common contributing factors were shortcomings of communication and flow of information. Risk classification showed only one AE to be of moderate risk, and all others were perceived as irrelevant or minor. Conclusions PSI in DMFR are only rarely reported, and mostly, they are perceived of causing little or no harm. We detected a great difference in reporting activity between primary and secondary healthcare workers, but the underlaying causes remain unclear.

Summary Purpose Black Bone (BB) magnetic resonance imaging (MRI) is a nonionizing imaging method and a recent alternative to computed tomography (CT) in the examination of cranial deformities. The purpose of this study was to compare BB-MRI and routine 3D-CT in the preoperative evaluation of patients with craniosynostosis. Methods At our center, we have routinely performed preoperative CT of the skull and brain MRI for patients with clinical suspicion of craniosynostosis. We recently changed our MRI protocol into one that includes sequences for the evaluation of both brain anatomy and skull bone and sutures by BB-MRI. A semi-automatic skull segmentation algorithm was developed to facilitate visualization. Both BB-MRI and 3D-CT were performed on 9 patients with clinical craniosynostosis, and the images were evaluated by two craniofacial surgeons, one pediatric neurosurgeon, and two neuroradiologists. Results We obtained informative 3D images using BB-MRI. Six (6/9) patients had scaphocephaly, 1 (1/9) patient had unicoronal synostosis, and 2 (2/9) patients had lambdoid synostosis. The affected synostotic sutures could be identified both by BB-MRI and by 3D-CT in all patients. Intra-rater and inter-rater reliability for rating the calvarial sutures was high. However, the reliability for rating the intracranial impressions was low by both imaging methods. Conclusion BB-MRI is an alternative to 3D-CT in the preoperative evaluation of patients with craniosynostosis. BB-MRI provides information not only on cranial sutures and intracranial impressions but also on the brain structure in one imaging session. This method can replace ionizing radiation-based methods in analyzing skull deformities.

Objective: Previous studies suggest that daily music listening can aid stroke recovery, but little is known about the stimulus-dependent and neural mechanisms driving this effect. Building on neuroimaging evidence that vocal music engages extensive and bilateral networks in the brain, we sought to determine if it would be more effective for enhancing cognitive and language recovery and neuroplasticity than instrumental music or speech after stroke. Methods: Using data pooled from two single-blind randomized controlled trials in stroke patients (N = 83), we compared the effects of daily listening to self-selected vocal music, instrumental music, and audiobooks during the first 3 poststroke months. Outcome measures comprised neuropsychological tests of verbal memory (primary outcome), language, and attention and a mood questionnaire performed at acute, 3-month, and 6-month stages and structural and functional MRI at acute and 6-month stages. Results: Listening to vocal music enhanced verbal memory recovery more than instrumental music or audiobooks and language recovery more than audiobooks, especially in aphasic patients. Voxel-based morphometry and resting-state and task-based fMRI results showed that vocal music listening selectively increased gray matter volume in left temporal areas and functional connectivity in the default mode network. Interpretation: Vocal music listening is an effective and easily applicable tool to support cognitive recovery after stroke as well as to enhance early language recovery in aphasia. The rehabilitative effects of vocal music are driven by both structural and functional plasticity changes in temporoparietal networks crucial for emotional processing, language, and memory.

BACKGROUND AND PURPOSE: Aspartylglucosaminuria is a rare lysosomal storage disorder that causes slowly progressive, childhood-onset intellectual disability and motor deterioration. Previous studies have shown, for example, hypointensity in the thalami in patients with aspartylglucosaminuria on T2WI, especially in the pulvinar nuclei. Susceptibility-weighted imaging is a neuroimaging technique that uses tissue magnetic susceptibility to generate contrast and is able to visualize iron and other mineral deposits in the brain. SWI findings in aspartylglucosaminuria have not been reported previously. MATERIALS AND METHODS: Twenty-one patients with aspartylglucosaminuria (10 girls; 7.4–15.0 years of age) underwent 3T MR imaging. The protocol included an SWI sequence, and the images were visually evaluated. Thirteen patients (6 girls, 7.4–15.0 years of age) had good-quality SWI. Eight patients had motion artifacts and were excluded from the visual analysis. Thirteen healthy children (8 girls, 7.3–14.1 years of age) were imaged as controls. RESULTS: We found a considerably uniform distribution of decreased signal intensity in SWI in the thalamic nuclei in 13 patients with aspartylglucosaminuria. The most evident hypointensity was found in the pulvinar nuclei. Patchy hypointensities were also found especially in the medial and anterior thalamic nuclei. Moreover, some hypointensity was noted in globi pallidi and substantia nigra in older patients. The filtered-phase images indicated accumulation of paramagnetic compounds in these areas. No abnormal findings were seen in the SWI of the healthy controls. CONCLUSIONS: SWI indicates accumulation of paramagnetic compounds in the thalamic nuclei in patients with aspartylglucosaminuria. The finding may raise the suspicion of this rare disease in clinical practice.

Ionising radiation exposure is especially harmful to brain development. The purpose of this study was to evaluate whether black-bone (BB) magnetic resonance imaging (MRI), a non-ionising imaging method, offers an alternative to ionising imaging methods such as computed tomography (CT) in the examination of cranial deformities. From 2012 to 2014, a total of 408 children were referred to the Craniofacial Centre at the Helsinki University Hospital for further examination due to flatness of the posterior skull. Fifteen of these patients required further diagnostic imaging. To avoid ionising radiation, we used an MRI protocol that included sequences for evaluation of both brain anatomy and skull bone and sutures by BB-MRI. A semi-automatic skull segmentation algorithm was developed to facilitate the visualisation. Two patients with scaphocephaly were included in the study to confirm the ability to differentiate synostosis with BB-MRI. We obtained informative 3D images using BB-MRI. Seven patients (7/15, 46.7%) had plagiocephaly on the right side and seven on the left side (7/15, 46.7%). One patient (1/15, 6.7%) had symmetric posterior flatness affecting both sides. Neither structural nor signal-intensity alterations of the brain were detected in visual analysis. BB-MRI provides an alternative to CT when imaging craniofacial deformities. BB-MRI provides not only high-quality 3D-reconstructed imaging of the bony structures and sutures but also information on brain structure in one imaging session. With further development, this method could replace ionising radiation-based methods in analysing deformities of the skull.

Aspartylglucosaminuria is an inherited, lysosomal storage disease causing progressive decline in cognitive and motor functions. The aim of this study was to evaluate volumes of subcortical gray matter structures and white matter microstructure in aspartylglucosaminuria in adolescence in a longitudinal study for the first time.A boy with aspartylglucosaminuria and his healthy twin brother were imaged twice with a 3.0 T MRI scanner at the ages of 10 and 15 years. Subcortical gray matter structure volumes were measured using an atlas-based automatic method, and diffusion tensor imaging was used to evaluate the white matter microstructure of the corpus callosum and the thalamocortical pulvinar tracts.The subcortical gray matter structures were smaller at onset and diminished at follow-up in the affected twin, with the exception of the amygdala which was larger and remained the size. The largest difference in volume between the twins was found in the thalami. The total gray and white matter volumes decreased in the affected twin. In diffusion tensor imaging analysis, the fractional anisotropy was decreased at onset in the affected twin compared to the healthy brother in the evaluated tracts. The axial, radial and mean diffusivity values were increased in the affected twin. The difference between the twins increased slightly at follow-up.The findings suggest that volumetric measurements and diffusion tensor imaging based microstructural analysis may be useful modalities for monitoring disease progression and response to emerging treatment in aspartylglucosaminuria, but further studies with more subjects are necessary to confirm the results.

We investigated cerebral processing of audiovisual speech stimuli in humans using functional magnetic resonance imaging (fMRI). Ten healthy volunteers were scanned with a 'clustered volume acquisition' paradigm at 3 T during observation of phonetically matching (e.g., visual and acoustic /y/) and conflicting (e.g., visual /a/ and acoustic /y/) audiovisual vowels. Both stimuli activated the sensory-specific auditory and visual cortices, along with the superior temporal, inferior frontal (Broca's area), premotor, and visual-parietal regions bilaterally. Phonetically conflicting vowels, contrasted with matching ones, specifically increased activity in Broca's area. Activity during phonetically matching stimuli, contrasted with conflicting ones, was not enhanced in any brain region. We suggest that the increased activity in Broca's area reflects processing of conflicting visual and acoustic phonetic inputs in partly disparate neuron populations. On the other hand, matching acoustic and visual inputs would converge on the same neurons.

Observing a speaker's articulatory gestures can contribute considerably to auditory speech perception. At the level of neural events, seen articulatory gestures can modify auditory cortex responses to speech sounds and modulate auditory cortex activity also in the absence of heard speech. However, possible effects of attention on this modulation have remained unclear. To investigate the effect of attention on visual speech‐induced auditory cortex activity, we scanned 10 healthy volunteers with functional magnetic resonance imaging (fMRI) at 3 T during simultaneous presentation of visual speech gestures and moving geometrical forms, with the instruction to either focus on or ignore the seen articulations. Secondary auditory cortex areas in the bilateral posterior superior temporal gyrus and planum temporale were active both when the articulatory gestures were ignored and when they were attended to. However, attention to visual speech gestures enhanced activity in the left planum temporale compared to the situation when the subjects saw identical stimuli but engaged in a nonspeech motion discrimination task. These findings suggest that attention to visually perceived speech gestures modulates auditory cortex function and that this modulation takes place at a hierarchically relatively early processing level. Hum Brain Mapp, 2005. © 2005 Wiley‐Liss, Inc.

highlights In 46 healthy newborns, activity from both the primary (SI) and secondary somatosensory cortices (SII) was detectable with magnetoencephalography to tactile stimulation of the contralateral index finger dur- ing quiet sleep (QS). No significant interhemispheric differences in the responses from SI or SII existed. Within the neonatal period (postmenstrual age (PMA) between 37 and 44 weeks) PMA, height, or gen- der did not significantly affect the latency or strength of the somatosensory evoked magnetic fields (SEFs). abstract Objective: To determine interhemispheric differences and effect of postmenstrual age (PMA), height, and gender on somatosensory evoked magnetic fields (SEFs) from the primary (SI) and secondary (SII) somatosensory cortices in healthy newborns. Methods: We recorded SEFs to stimulation of the contralateral index finger (right in 46 and left in 12) healthy fullterm newborns and analyzed the magnetic responses with equivalent current dipoles. Results: Activity from both the SI and SII was consistently detectable in the contralateral hemisphere of the newborns during quiet sleep. No significant interhemispheric differences existed in SI or SII response peak latencies, source strengths, or location (n = 8, quiet sleep). SI or SII response peak latency or source strength were not significantly affected by PMA, height, or gender. Conclusions: During the neonatal period (PMA 37-44 weeks), activity from the contralateral SI and SII can be reliably evaluated with MEG. The somatosensory responses are similar in the left and right hemi- spheres and no corrections for exact PMA, height, or gender are necessary for interpreting the results. However, the evaluation should be conducted in quiet sleep. Significance: The reproducibility of the magnetic SI and SII responses suggests clinical applicability of the presented MEG method.

Objective Drug smuggling in the gastrointestinal tract has soared within the last 20 years. Though illegal substances in the gastrointestinal tract can be visualized with ultrasound, MRI and CT, the abdominal radiograph has by far remained the most frequently used way of detecting smuggled drugs. The purpose of the study was to evaluate the inter-radiologist interpretation error and the reliability of the abdominal radiograph in detecting smuggled drugs. Materials and methods A total of 279 abdominal radiographs of suspected smugglers were classified by three radiologists as clearly positive or negative for drug smuggling. All available information about the cases was collected from the customs officers and police. Results Out of these cases 203 (73%) were interpreted as negative and 35 (13%) as positive by all three radiologists. In 86% of the cases there was, therefore, an inter-radiological agreement in interpreting the images. In 41 (14%) cases, however, there was an inter-radiologist disagreement. Kappa-value for inter-observer variability was 0.70. Conclusions In up to a seventh of the abdominal radiographs the interpretation can be challenging even for an experienced radiologist. False positive interpretation can lead to innocent passengers being detained in vain. As negatively interpreted images usually result in releasing of the suspect, there is no way of knowing how many false negative occur. This makes the abdominal radiograph a suboptimal examination, and low dose CT should be considered as the screening modality for gastrointestinal drug smugglers.

Emotion plays a significant role in goal‐directed behavior, yet its neural basis is yet poorly understood. In several psychological models the cardinal dimensions that characterize the emotion space are considered to be valence and arousal. Here 3T functional magnetic resonance imaging (fMRI) was used to reveal brain areas that show valence‐ and arousal‐dependent blood oxygen level dependent (BOLD) signal responses. Seventeen healthy adults viewed pictures from the International Affective Picture System (IAPS) for brief 100 ms periods in a block design paradigm. In many brain regions BOLD signals correlated significantly positively with valence ratings of unpleasant pictures. Interestingly, partly in the same regions but also in several other regions BOLD signals correlated negatively with valence ratings of pleasant pictures. Therefore, there were several areas where the correlation across all pictures was of inverted U‐shape. Such correlations were found bilaterally in the dorsolateral prefrontal cortex (DLPFC), dorsomedial prefrontal cortex (DMPFC) extending to anterior cingulate cortex (ACC), and insula. Self‐rated arousal of those pictures which were evaluated to be unpleasant correlated with BOLD signal in the ACC, whereas for pleasant pictures arousal correlated positively with the BOLD signal strength in the right substantia innominata. We interpret our results to suggest a major division of brain mechanisms underlying affective behavior to those evaluating stimuli to be pleasant or unpleasant. This is consistent with the basic division of behavior to approach and withdrawal, where differentiation of hostile and hospitable stimuli is crucial. Hum Brain Mapp, 2010. © 2009 Wiley‐Liss, Inc.

Recent evidence on amusia suggests that our ability to perceive music might be based on the same neural resources that underlie other higher cognitive functions, such as speech perception and spatial processing. We studied the neural correlates of acquired amusia by performing extensive neuropsychological assessments on 53 stroke patients with a left or right hemisphere middle cerebral artery (MCA) stroke 1 week, 3 months, and 6 months after the stroke. In addition, structural magnetic resonance imaging (MRI) was performed on all patients 1 week and 6 months post-stroke. Based on their performance on a shortened version of the Montreal Battery of Evaluation of Amusia (MBEA), the patients were classified as amusic (n=32) or non-amusic (n=21). MRI results showed that the incidence of auditory cortex and frontal lobe damage was significantly higher in the amusic group than in the non-amusic group, but the two groups did not differ in respect to lesion laterality. Cognitively, amusia was associated with general deficits in working memory and learning, semantic fluency, executive functioning, and visuospatial cognition, as well as hemisphere-specific deficits in verbal comprehension, mental flexibility, and visuospatial attention (unilateral spatial neglect). Moreover, the recovery of music perception ability was related to the recovery of verbal learning, visuospatial perception and attention, and focused attention, especially in amusic patients. Together, these results suggest the ability to perceive music is closely linked to other higher cognitive functions.

We studied the relationship between musical and cognitive deficits by testing middle cerebral arterial (MCA) stroke patients (n= 53) with a shortened version of the Montreal Battery of Evaluation of Amusia (MBEA) and an extensive neuropsychological test battery. Results showed that amusic patients (n= 32) had more severe cognitive deficits, especially in working memory and executive functioning, than did non-amusic patients (n= 21), and the severity of amusia also correlated with attention deficits. These findings thus suggest that domain-general attention, executive, and working memory processes are associated with amusia after stroke.

Reported here is the 30-year follow-up of a patient, diagnosed with juvenile neuronal ceroid lipofuscinosis, who was compound heterozygous for the common 1-kb deletion and the missense mutation p.Glu295Lys in the CLN3 gene. Visual failure was noticed at 6 years of age, but thereafter disease progression was atypical. Polyneuropathy and cerebellar signs were observed after age 20, and epilepsy and slight mental decline after age 35. From then on, there was rapid deterioration, and the patient died at age 39. This case highlights the importance of exact genotyping for disease course prediction and management.

Objective To explore how growth measurements and attainment of developmental milestones in early childhood reflect the need for full-time special education (SE). Methods After stratification in this population-based study, 900 pupils in full-time SE groups (age-range 7–16 years, mean 12 years 8 months) at three levels and 301 pupils in mainstream education (age-range 7–16, mean 12 years 9 months) provided data on height and weight from birth to age 7 years and head circumference to age 1 year. Developmental screening was evaluated from age 1 month to 48 months. Statistical methods included a general linear model (growth measurements), binary logistic regression analysis (odds ratios for growth), and multinomial logistic regression analysis (odds ratios for developmental milestones). Results At 1 year, a 1 standard deviation score (SDS) decrease in height raised the probability of SE placement by 40%, and a 1 SDS decrease in head size by 28%. In developmental screening, during the first months of life the gross motor milestones, especially head support, differentiated the children at levels 0–3. Thereafter, the fine motor milestones and those related to speech and social skills became more important. Conclusion Children whose growth is mildly impaired, though in the normal range, and who fail to attain certain developmental milestones have an increased probability for SE and thus a need for special attention when toddlers age. Similar to the growth curves, these children seem to have consistent developmental curves (patterns).

Selective auditory attention powerfully modulates neural activity in the human auditory cortex (AC). In contrast, the role of attention in subcortical auditory processing is not well established. Here, we used functional MRI (fMRI) to examine activation of the human inferior colliculus (IC) during strictly controlled auditory attention tasks. The IC is an obligatory midbrain nucleus of the ascending auditory pathway with diverse internal and external connections. The IC also receives a massive descending projection from the AC, suggesting that cortical processes affect IC operations. In this study, 21 subjects selectively attended to left-ear or right-ear sounds and ignored sounds delivered to the other ear. IC activations depended on the direction of attention, indicating that auditory processing in the human IC is not only determined by acoustic input but also by the current behavioral goals.

Increased survival of extremely low birth weight infants has led to a need for new prognostic methods to predict possible future neurological impairment. We investigated the early development of the somatosensory system by recording the somatosensory evoked magnetic fields (SEFs) during natural sleep at fullterm age in 16 very prematurely born infants and 16 healthy newborns born at term. The purpose was to determine possible changes in the function of the somatosensory cortex in the prematurely born infants by comparing the latency, strength, location and morphology of the SEFs with those of healthy fullterm newborns. We recorded reliable SEFs in all patients and controls. The equivalent current dipole (ECD) strength of the first cortical response, M60, was significantly lower in the patients. Otherwise, the general morphology and latency of the SEFs were similar in the two groups of babies. The similar response latencies in the two groups indicate normally developed conduction in the somatosensory system of the prematurely born infants. The attenuated ECD strength may reflect weaker synchrony in firing or a smaller number of the cortical neurons activated by the somatosensory stimulation. At the individual level, in four of the preterm infants, a later M200 response was not present or could not be modeled: all of them had lesions of the underlying hemisphere depicted by ultrasound and magnetic resonance imaging.

Summary Objectives There is evidence suggesting that secondhand smoke (SHS) exposure is causally linked to adverse respiratory effects. We examined the relations between the exposure to SHS and radiological signs in chest high-resolution computed tomography (HRCT). Methods Asbestos-exposed workers ( n =633) were imaged with HRCT, primarily to investigate potential occupational lung disease. After excluding current smokers, the study population included 361 ex- and 141 never-smokers. They answered a questionnaire on occupational exposures, smoking habits and SHS exposure. HRCT images were assessed for emphysema, ground-glass, irregular/linear and rounded opacities, honeycombing and several other signs. Regression analyses were adjusted for asbestos exposure, ex-smoking, age, body mass index and potential reader effect. Results Due to missing data the multivariate analyses were restricted to 310 participants aged 47.5–87.0 years. Their lifetime SHS exposure ranged between 0 and 193.5 pack-years (mean 23.5), and exposure in the past 12 months 0–30 packs (0.43). Total ( B =0.005, 95% confidence intervals (95% CI) 0.002–0.008, p =0.000) and workplace ( B =0.006, 95% CI 0.003–0.009, p =0.001) cumulative SHS exposures were significantly related to ground-glass opacities. Total SHS exposure in the last 12 months ( B =0.027, 95% CI 0.000–0.054, p =0.048) and workplace exposure ( B =0.027, 95% CI 0.000–0.054, p =0.048) were also significantly related to ground-glass opacities. Positive effects of SHS were also detected on irregular/linear opacities. Conclusions SHS exposure in the last 12 months and over lifetime significantly increases ground-glass opacity in HRCT, suggesting an early or subclinical desquamative interstitial pneumonia/respiratory bronchiolitis. This study further supports that SHS has adverse effects on the lungs that can be detected by X-ray methods.

Hemodynamic activity in occipital, temporal, and parietal cortical areas were recently shown to correlate across subjects during viewing of a 30-minute movie clip. However, most of the frontal cortex lacked between-subject correlations. Here we presented 12 healthy naïve volunteers with the first 72 minutes of a movie (“Crash”, 2005, Lions Gate Films) outside of the fMRI scanner to involve the subjects in the plot of the movie, followed by presentation of the last 36 minutes during fMRI scanning. We observed significant between-subjects correlation of fMRI activity in especially right hemisphere frontal cortical areas, in addition to the correlation of activity in temporal, occipital, and parietal areas. It is possible that this resulted from the subjects following the plot of the movie and being emotionally engaged in the movie during fMRI scanning. We further show that probabilistic independent component analysis (ICA) reveals meaningful activations in individual subjects during natural viewing.

Cognitive dysfunctions may be a significant factor in drug-seeking behavior, reducing the efficiency of rehabilitation in opioid dependence. Neurophysiological basis of these dysfunctions is poorly understood. 21 opioid-dependent patients and 15 healthy controls with no experience of illicit drugs were studied with simultaneous electroencephalography (EEG) and magnetoencephalography (MEG). Among opioid dependents 15 were benzodiazepine co-dependent. In a passive oddball paradigm, a train of 700-Hz standard tones (80%), presented to the left ear, was occasionally interrupted by infrequent deviants, which were either 600-Hz or 400-Hz pure tones or complex novel sounds. The auditory evoked potentials (AEP) and fields (AEF) were analyzed. The strength of the N1m dipoles was enhanced in patients with benzodiazepine co-dependence, but the latency of the response or the source location was not changed. A delay of mismatch negativity (MMN) response of novel tones in EEG, and delay of P3am response on the contralateral hemisphere to stimulated ear in MEG in opioid-dependent patients were observed. There were no differences in source locations or strengths of the dipoles for P1m, MMNm, and P3am determined using equivalent current dipoles. There were no group differences in EEG amplitude measures. In conclusion, our results suggest delayed pre-attentive auditory processing of novel information in opioid dependence. Benzodiazepine co-dependence modulated N1m response.

Cathepsin D (CTSD; EC 3.4.23.5) is essential for normal development and/or maintenance of neurons in the central nervous system: its deficiency causes a devastating neurological disorder with severely shortened life span in man, sheep and mouse. Neuropathologically, the CTSD deficiencies are characterized by selective neuronal degeneration, gliosis and accumulation of autofluorescent proteinaceous storage material in neurons. Our aim was to study the dynamics behind the pathological alterations occurring in the brains of CTSD-deficient (CTSD-/-) mice by using in vivo magnetic resonance imaging (MRI) and histology. In order to do this, we measured T(2) signal intensity (SI), apparent diffusion coefficient, area and volume of multiple brain structures from MR images acquired using T(2)-, T(1)- and diffusion-weighted sequences at three time points during disease progression. MRI revealed no differences in the brains between CTSD-/- and control mice at postnatal day 15+/-1 (P15+/-1), representing an initial stage of the disease. In the intermediate stage of the disease, P19(+/-1), SI alterations in the thalami of the affected mice became evident in both T(1)- and T(2)-weighted images. The terminal stage of the disease, P25, was characterized by marked alterations in the T(2) SI, apparent diffusion coefficient and volume of multiple brain structures in CTSD-/- mice. In addition, manganese enhanced high-resolution T(1)-weighted 3D sequences (MEMRI) and histological stainings revealed that the hyperintense signal areas in MEMRI matched perfectly with areas of microglial activation in the brains of CTSD-/- mice at the terminal disease stage. In conclusion, the SI alterations in the thalami of CTSD-/- mice preceded other changes, and the degenerative process was greatly enhanced at the age P19(+/-1), leading to severely reduced brain volume in just 6 days.

Withdrawal may be a natural model to study craving and compulsive drug seeking, since craving can be viewed as a conditioned dysphoric state. It has been suggested that functional connectivity between brain areas may be of major value in explaining excessive craving and compulsive drug seeking by providing essential link between psychological and biological processes. Considering that withdrawal initiates a widespread activation of cortical regions responsible for compulsive drug seeking and desire for the drug, we predict that withdrawal would result in a significant increase in functional cortical connectivity. We applied the novel operational architectonics approach that enables us to estimate both local and remote functional cortical connectivity by means of EEG structural synchrony measure. In 13 withdrawal opioid-dependent patients we found the evidence that local and remote cortical functional connectivity was indeed significantly enhanced (for both alpha and beta frequency oscillations). Additionally, statistical relationship between functional connectivity and the severity of opioid withdrawal has been found.

Purpose: To evaluate the brains of newborns exposed to buprenorphine prenatally. Material and Methods: Seven neonates followed up antenatally in connection with their mothers' buprenorphine replacement therapy underwent 1.5T magnetic resonance imaging (MRI) of the brain before the age of 2 months. The infants were born to heavy drug abusers. Four mothers were hepatitis C positive, and all were HIV negative. All mothers smoked tobacco and used benzodiazepines. All pregnancies were full term, and no perinatal asphyxia occurred. All but one neonate had abstinence syndrome and needed morphine replacement therapy. Results: Neither structural abnormalities nor abnormalities in signal intensity were recorded. Conclusion: Buprenorphine replacement therapy does not seem to cause any major structural abnormalities of the brain, and it may prevent known hypoxic-ischemic brain changes resulting from uncontrolled drug abuse. Longitudinal studies are needed to assess possible abnormalities in the brain maturation process.

The objective of the present study is to determine the feasibility of chest computed tomography (CT) in screening for lung cancer among asbestos-exposed workers. In total, 633 workers were included in the present study and were examined with chest radiography and high-resolution CT (HRCT). A total of 180 current and ex-smokers (cessation within the previous 10 yrs) were also screened with spiral CT. Noncalcified lung nodules were considered positive findings. The incidental CT findings not related to asbestos exposure were registered and further examined when needed. Noncalcified lung nodules were detected in 86 workers. Five histologically confirmed lung cancers were found. Only one of the five cancers was also detected by plain chest radiography and three were from the group of patients with a pre-estimated lower cancer probability. Two lung cancers were stage Ia and were radically operated. In total, 277 individuals presented 343 incidental findings of which 46 required further examination. Four of these were regarded as clinically important. In conclusion, computed tomography and high-resolution computed tomography proved to be superior to plain radiography in detecting lung cancer in asbestos-exposed workers with many confounding chest findings. The numerous incidental findings are a major concern for future screenings, which should be considered for asbestos-exposed ex-smokers and current smokers.

Although researchers now have a working knowledge of key brain structures involved in realization of actions of substance abuse and addiction, deeper understanding will require examination of network interactions between cortical neuronal assemblies and their subcortical tails in the effects of opioid dependence.Given that repeated exposure to opiates initiates a widespread reorganization of cortical regions, we predict that opioid dependence would result in a considerable reorganization of local and remote functional connectivity in the neocortex.We applied the novel operational architectonics approach that enables us to estimate two local and remote functional cortex connectivities by means of electroencephalogram structural synchrony measure.In 22 opioid-dependent patients, we found the evidence that brain functional connectivity was indeed disrupted by chronic opioid abuse (i.e., the local functional connectivity increased and remote functional connectivity decreased in opioid abusers). This significant difference between "opioid" and "control" populations was the same for alpha and beta frequency bands. Additionally, significant negative relations between duration (years) of daily opioid abuse and the number/strength of functional connections in the posterior section of the cortex were found.

We presented phonetically matching and conflicting audiovisual vowels to 10 dyslexic and 10 fluent-reading young adults during “clustered volume acquisition” functional magnetic resonance imaging (fMRI) at 3 T. We further assessed co-variation between the dyslexic readers' phonological processing abilities, as indexed by neuropsychological test scores, and BOLD signal change within the visual cortex, auditory cortex, and Broca's area. Both dyslexic and fluent readers showed increased activation during observation of phonetically conflicting compared to matching vowels within the classical motor speech regions (Broca's area and the left premotor cortex), this activation difference being more extensive and bilateral in the dyslexic group. The between-group activation difference in conflicting > matching contrast reached significance in the motor speech regions and in the left inferior parietal lobule, with dyslexic readers exhibiting stronger activation than fluent readers. The dyslexic readers' BOLD signal change co-varied with their phonological processing abilities within the visual cortex and Broca's area, and to a lesser extent within the auditory cortex. We suggest these findings as reflecting dyslexic readers' greater use of motor-articulatory and visual strategies during phonetic processing of audiovisual speech, possibly to compensate for their difficulties in auditory speech perception.

Background. Recreational drug abuse is one of the most important risk factors for stroke in young adults. Abuse of opiates may lead to severe acute neurologic problems due to ischemia or hemorrhage. In contrast, their minor effects on brain structures are not well established. We evaluated brain magnetic resonance images (MRI) of opiate-dependent subjects who had no major neurologic symptoms or psychiatric disorder. Methods. Seventeen opiate-dependent patients and 17 controls underwent 1.5 T MRI. Any abnormalities in signal intensity of the brain were recorded. Areas of vermis, corpus callosum, and midline internal skull surface (MISS) were measured from midline sagittal slice. To evaluate size of cortical sulci, sylvian fissures, and ventricles, axial images were compared with standard sets of reference images. In addition, bifrontal and sylvianfissure ratios were measured. Results. Only one patient had a small subcortical post-traumatic lesion; otherwise, gray and white matter showed normal signal intensities. Opiate-dependent subjects had significantly wider sylvian fissures (p 0.008, Mann-Whitney U) and larger ventricles (p 0.04) than controls. Bifrontal and sylvian-fissure ratios were significantly higher in patient group than in controls (p 0.013 and p 0.005, respectively). Conclusions. No signs of brain pathology of vascular origin were found. From the clinical point of view, we want to emphasize that in the first acute neurologic attack of opiatedependent patients, any abnormal signal intensity in MRI is most probably associated with the patient’s current situation. Sylvian fissures and ventricles were wider in opiatedependent subjects than in controls, which may be related to brain atrophy located especially in frontal and temporal lobes. 2004 IMSS. Published by Elsevier Inc.

Background: A new leukoencephalopathy with brainstem and spinal cord involvement and high brain lactate was recently defined. The authors describe five new patients with this entity. Methods: Brain MRI was performed in all patients and spinal MRI and proton magnetic resonance spectroscopy ( 1 H-MRS) in four patients. Laboratory examinations ruled out classic leukodystrophies. Results: MRI showed signal abnormalities in the periventricular and deep white matter, in the pyramidal tracts, mesencephalic trigeminal tracts, in the cerebellar connections, and in dorsal columns of the spinal cord. MRS showed decreased N -acetylaspartate and increased lactate in the white matter of all patients. In one patient choline-containing compounds were elevated. A slowly progressive sensory ataxia and tremor manifested at the age of 3 to 16 years and distal spasticity in adolescence. One 13-year-old patient was asymptomatic. Conclusions: A slowly progressive sensory ataxia is a typical feature in this new leukodystrophy. MRS favors a primary axonal degeneration.

The neuronal ceroid lipofuscinoses (NCLs) are among the most severe inherited progressive neurodegenerative disorders of children. The purpose of this study was to compare the in vivo 1.5-T 1H magnetic resonance (MR) and ex vivo 14.3-T high-resolution (HR) magic angle spinning (MAS) 1H MR brain spectra of patients with infantile (CLN1) and juvenile (CLN3) types of NCL, to obtain detailed information about the alterations in the neuronal metabolite profiles in these diseases and to test the suitability of the ex vivo HR MAS 1H MRS technique in analysis of autopsy brain tissue. Ex vivo spectra from CLN1 autopsy brain tissue (n = 9) significantly differed from those of the control (n = 9) and CLN3 (n = 5) groups, although no differences were found between the CLN3 and the control groups. Principal component analysis of ex vivo data showed that decreased levels of N-acetylaspartate (NAA), γ-aminobutyric acid (GABA), glutamine, and glutamate as well as increased levels of inositols characterized the CLN1 spectra. Also, the intensity ratio of lipid methylene/methyl protons was decreased in spectra of CLN1 brain tissue compared with CLN3 and control brain tissue. In concordance with the ex vivo data, the in vivo spectra of late-stage patients with CLN1 (n = 3) revealed a dramatic decrease of NAA and a proportional increase of myo-inositol and lipids compared with control subjects. Again, the spectra of patients with CLN3 (n = 13) did not differ from those of controls (n = 15). In conclusion, the ex vivo and in vivo spectroscopic findings were in good agreement within all analyzed groups and revealed significant alterations in metabolite profiles in CLN1 brain tissue but not in CLN3 compared with controls. Furthermore, HR MAS 1H MR spectra facilitated refined detection of neuronal metabolites, including GABA, and composition of lipids in the autopsy brain tissue of NCL patients. © 2004 Wiley-Liss, Inc.

The etiology of Asperger syndrome is essentially unknown, but abnormality of the dopamine system has been shown in clinically overlapping disorders. The present study was designed to investigate the presynaptic dopamine function in Asperger syndrome. Eight healthy, drug-free males with Asperger syndrome and five healthy male controls were examined with positron emission tomography using 6-[18F]fluoro-L-DOPA ([18F]FDOPA) as a tracer. In the Asperger syndrome group, the [18F]FDOPA influx (Ki) values were increased in the striatum, i.e. in the putamen and caudate nucleus and in the frontal cortex. The results indicate that the dopamine system is affected in subjects with Asperger syndrome. Partially similar results have also been obtained in schizophrenia, suggesting an overlap not only of the clinical features but also of pathogenesis.

Purpose To study brain MRI findings in patients with 18q− syndrome and to correlate these findings with the results of the molecular breakpoint analysis. Materials and Methods Brain MR images of 17 patients with 18q− syndrome were evaluated. Segregation analysis was performed with 15 microsatellite markers to determine the deletion breakpoints and whether the deletion included the myelin basic protein (MBP) gene. Results One patient had an interstitial deletion of 18q which spared the MBP gene. He was the only one with normal brain MRI. All 16 patients with deletions including the MBP gene had abnormal white matter in MRI. The main finding was poor differentiation of gray and white matter on T2-weighted images due to increased white matter signal intensity. In addition, measured signal intensity of the white matter was significantly increased in patients compared with controls. Conclusions Poor differentiation of gray and white matter on T2-weighted images is the most typical MRI finding of the 18q− syndrome. These results support the postulation that abnormal myelination in 18q− syndrome is due to haploinsufficiency at or near the MBP locus. J. Magn. Reson. Imaging 2003;18:414–419. © 2003 Wiley-Liss, Inc.

Lack of theory of mind (ToM) has been considered to be a key feature in Asperger syndrome (AS). The main aim of the present study was to determine whether an exclusively auditory input of ToM stories activated the same brain areas as demonstrated previously using visual stimuli. Eight right-handed otherwise healthy men with AS and eight healthy right-handed male controls participated in a PET activation study using auditory given ToM stories and stories about physical events for induction. Both subjects with AS and controls showed increased activation in the occipitotemporal area bilaterally and in thalamus during ToM tasks. Both groups also showed activation in the medial frontal area during ToM tests.However, this activation was more intensive and extensive in the control group, especially when a more sensitive analysis method was used. As a group, unrelated to the tasks, the AS subjects showed increased activation of the cerebellum. It was concluded that the activation pattern was mainly in agreement with earlier studies using comparable stimuli administered differently. There was no support for a right hemisphere specific dysfunction.

Objectives : To examine in detail the activation of the primary (SI) and secondary (SII) somatosensory cortex in CLN5, the Finnish variant of late infantile neuronal ceroid lipofuscinoses (NCL). Methods : Somatory evoked magnetic fields were recorded with a 122-channel planar gradiometer in response to median nerve stimulation in 5 CLN5 patients (aged 8.8–16.7 years) and in 10 healthy age-matched controls. Results : The first two responses from contralateral SI, N20m and P35m, were 6–20 times stronger in the patients than in the controls. The morphology of the subsequent deflections from SI was abnormal in the patients: a prominent N45m was detected, while the normally present P60m deflection was missing. In 4 patients the contra- and in two patients the ipsilateral SII responses were also enlarged. Furthermore, the SII activation was detected at shorter latency in patients than in controls. Conclusions : At SI, CLN5 is associated with a selective enhancement of the early cortical responses. We propose that the enlargement of N20m most likely reflects increased synchronous input from thalamus, whereas the altered morphology of the following responses may reflect defective interneuronal inhibition at the cortex. The enlargement of SII responses shows that the imbalance between excitation and inhibition in CLN5 extends outside the primary somatosensory areas.

Summary Background and purpose The aim of this study was to identify characteristic 3.0 T brain MRI findings in patients with aspartylglucosaminuria (AGU), a rare lysosomal storage disorder. Previous AGU patient material imaged at 1.0 and 1.5 T was also re-evaluated. Materials and methods Twenty-five brain MRI examinations from 20 AGU patients were included in the study. Thirteen patients underwent a prospective 3.0 T MRI (5 male, 8 female, aged 9–45 years). Twelve examinations from nine patients (4 male, 5 female, aged 8–33 years) previously imaged at 1.0 or 1.5 T were re-evaluated. Two patients were included in both the prospective and the retrospective groups. Visual analysis of the T1- and T2-weighted images was performed by two radiologists. Results The previously reported signal intensity changes in T2-weighted images were visible at all field strengths, but they were more distinct at 3.0 T than at 1.0 or 1.5 T. These included signal intensity decrease in the thalami and especially in the pulvinar nuclei, periventricular signal intensity increase and juxtacortical high signal foci. Poor differentiation between gray and white matter was found in all patients. Some degree of cerebral and/or cerebellar atrophy and mild ventricular dilatation were found in nearly all patients. This study also disclosed various unspecific findings, including a higher than normal incidence of dilated perivascular spaces, arachnoid cysts, pineal cysts and mildly dilated cavum veli interpositi. Conclusion This study revealed particular brain MRI findings in AGU, which can raise the suspicion of this rare disease in clinical practice.

Aim Atypical sensory processing is common in children born extremely prematurely. We investigated sensory processing abilities in extremely low gestational age (ELGA) children and analysed associated neonatal risk factors, neuroanatomical findings and neurodevelopmental outcome. Methods We carried out a prospective study of 44 ELGA children, including 42 who had undergone brain magnetic resonance imaging (MRI) at term-equivalent age, when they were 2 years of corrected age. Their sensory processing abilities were assessed with the Infant/Toddler Sensory Profile questionnaire and their neurodevelopmental with a structured Hempel neurological examination, Griffiths Mental Developmental Scales and Bayley Scales of Infant and Toddler Development Third Edition. Results Sensory profiles were definitely or probably atypical (

Prior studies on extremely preterm infants have reported long-term prognostic value of absent secondary somatosensory cortex (SII) responses in magnetoencephalography (MEG) at term. The present work (i) further examines the potential added value of SII responses in neonatal neurological evaluation of preterm infants, and (ii) tests whether SII responses are detectable in routine neonatal electroencephalogram complemented with median nerve stimulation (EEG-SEP).Altogether 29 infants born28 gestational weeks underwent MEG, MRI, and neonatal neurological examination at term age, and Hempel neurological examination at 2-years corrected age. Term-age EEG-SEP was available for seven infants.While in neonatal neurological examination severely abnormal finding predicted unfavorable outcome in 2/2 infants, outcome was unfavorable also in 3/9 (33%) moderately abnormal and in 5/18 (28%) mildly abnormal/normal infants. Of these eight infants four had unilaterally absent SII responses in MEG, compared with only two of the 24 infants with favorable outcome. Furthermore, SII responses (when present in MEG) were also usually detectable in EEG-SEP.Complementing clinical EEG recording with SEP holds promise for valuable extension of neonatal neurophysiological assessment.Multimodal study of EEG and sensory evoked responses is informative, safe, and cheap, and it can be readily performed at bedside.

Objective: To study the effect of allogeneic hematopoietic stem cell transplantation (SCT) on the clinical course of infantile neuronal ceroid lipofuscinosis (INCL), a lysosomal storage disease. Background: INCL is a progressive encephalopathy with severe neuronal loss, especially in the cerebral and cerebellar cortex and retina. Autofluorescent lipopigments constitute the typical storage material in INCL. The disease is caused by recessive mutations in the palmitoyl protein thioesterase 1 (PPT1) gene. PPT1 is a depalmitoylating enzyme, which is transported to lysosomes through the mannose-6-phosphate receptor-mediated pathway, and participates in the lysosomal degradation of fatty acylated proteins. Methods: Three patients with INCL received transplants and were followed up after SCT at the Hospital for Children and Adolescents at the University of Helsinki. The first patient rejected the first graft at the age of 7 months and had mild symptoms of INCL at the second transplantation at 11 months. The two other patients were asymptomatic when they received their transplants at the age of 4 months. Results: PPT1 enzyme activity was normalized in peripheral leukocytes, but remained low in the CSF and resulted only in a mild and transient amelioration of the classic INCL. All patients who received transplants developed INCL by the age of 2 or 3 years. Conclusions: More experimental animal and cell culture studies are needed to determine the in vivo function of PPT1. SCT currently cannot be recommended as therapy for INCL.